Sickle Cell Anemia

Overview

What is sickle cell anemia?

Sickle cell anemia is a blood disease that affects red blood cells. Normal red blood cells are round. In people with sickle cell anemia, hemoglobin – a substance in red blood cells – becomes defective and causes the red blood cells to change shape. The faulty hemoglobin is called hemoglobin S (HgbS), and it replaces normal hemoglobin which is called hemoglobin A (HgbA). Over time, the red blood cells become rigid and shaped like crescent moons or sickles.

The sickle-shaped red blood cells:

  • Clog blood vessels, causing episodes of pain and cutting off oxygen to tissues and organs.
  • Get trapped in the spleen (an organ that gets rid of old cells) where they are destroyed. The body cannot replace the lost cells fast enough. As a result, the body has too few red blood cells, a condition known as anemia.

Sickle cell anemia is a serious disease that can require frequent hospital stays. Children and young adults can die from the disease.

Who gets sickle cell anemia?

In the United States, the disease occurs most often among African Americans (in about 1 of every 400 African American births) and among Hispanics of Caribbean ancestry (1 in every 1,000 to 1,400 Hispanic American children). Throughout the world, the disease is also found among people of Arabian, Greek, Italian, Sardinian, Turkish, Maltese, and southern Asian ancestry.

Is there a difference between sickle cell anemia and sickle cell trait?

Yes. A person can have a mixture of normal and faulty hemoglobin in their red blood cells without having sickle cell disease. This condition is called "sickle cell trait." People with sickle cell trait have enough normal hemoglobin in their red blood cells to prevent the cells from sickling. One in 12 African Americans in the United States has sickle cell trait.

It's important to remember that people with sickle cell trait do not have sickle cell disease. They also usually do not develop sickle cell disease, except in unusual circumstances. However, people with sickle cell trait can genetically pass the trait to their children. If two people with sickle cell trait have children together, there is a 1 in 4 chance that their children will have sickle cell anemia.

What are the chances that my child will be born with sickle cell anemia or sickle cell trait?

If you and your partner both have sickle cell trait, your child has a 25% chance of being born with sickle cell anemia. If only one of you has sickle cell trait, your child cannot be born with sickle cell anemia, but there is a 50% chance that your child will be born with sickle cell trait.

If one parent has sickle cell disease and one parent has sickle cell trait, there is a 50% chance that their children will be born with sickle cell disease.

Symptoms and Causes

How does a person get sickle cell anemia?

People with sickle cell anemia inherit the disease, which means that the disease is passed on to them by their parents as part of their genetic makeup. Parents cannot give sickle cell anemia to their children unless they both have the faulty hemoglobin in their red blood cells.

What are the symptoms and complications of sickle cell anemia?

  • Periods of pain that can last a few hours to a few days.
  • Blood clots.
  • Swelling in hands and feet.
  • Joint pain that resembles arthritis.
  • Chronic neuropathic pain (nerve pain).
  • Life-threatening infections.
  • Anemia (decrease in red blood cells).

Diagnosis and Tests

How can I know if I have sickle cell trait?

Your healthcare provider can perform a special blood test to tell if you have sickle cell anemia or sickle cell trait. You may decide to have this test before you plan to have children.

In many states, the law requires newborn babies to be tested for sickle cell disease, regardless of their ethnic background. The testing is done right away so that children born with sickle cell disease can receive treatment to protect them against life-threatening infections. These children need to be followed very closely by a healthcare provider.

Management and Treatment

Can sickle cell anemia be cured?

No. As of today, there's no cure for sickle cell anemia. However, there are treatments that have reduced the death rate among children and the levels of pain caused by the disease.

If your baby has sickle cell anemia, your healthcare provider will explain what you can do to help your child live a normal life. Your baby may need to take medicine by the mouth for up to 10 years to prevent life-threatening infections. Later in life, care focuses more on managing pain.

Living With

How can I manage my pain if I have sickle cell anemia?

Acute pain can occur during a vaso-occlusive crisis (VOC). This happens when the sickle-shaped blood cells block the flow of blood in small vessels. The VOC can lead to tissue damage and pain. This type of pain should be treated as a medical emergency.

Some patients develop chronic pain, which is pain that lasts for more than 3 to 6 months. The exact mechanism that causes chronic pain in some patients and not others is not known. The treatment of the chronic pain should be tailored to the specific type of pain that is being experienced. Wokring with a pain management doctor can help you manage your pain by applying different methods. The goal is to use the least of amount of medication and gain the greatest amount of function.

Resources

Where can I get more information about sickle cell anemia?

For more information, contact:

American Sickle Cell Anemia Association

10900 Carnegie Avenue

Cleveland, Ohio 44106

216.229.8600

March of Dimes Foundation

1275 Mamaroneck Avenue

White Plains, New York 10605

914.997.4488

(Or contact your local March of Dimes.)

Sickle Cell Disease Association of America

231 East Baltimore Street, Ste 800

Baltimore, Maryland 21202

800.421.8453

Last reviewed by a Cleveland Clinic medical professional on 05/31/2019.

References

  • National Heart, Lung, and Blood Institute. . Accessed 6/4/2019.Sickle Cell Disease (https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease)
  • Centers for Disease Control and Prevention. Accessed 6/4/2019.What is Sickle Cell Disease? (https://www.cdc.gov/ncbddd/sicklecell/facts.html)
  • Centers for Disease Control and Prevention. Accessed 6/4/2019.What is Sickle Cell Trait? (https://www.cdc.gov/ncbddd/sicklecell/traits.html)
  • Natrajan K, Kutlar A. Natrajan K, Kutlar A Natrajan, Kavita, and Abdullah Kutlar.Disorders of Hemoglobin Structure: Sickle Cell Anemia and Related Abnormalities. In: Kaushansky K, Lichtman MA, Prchal JT, Levi MM, Press OW, Burns LJ, Caligiuri M. Kaushansky K, Lichtman M.A., Prchal J.T., Levi M.M., Press O.W., Burns L.J., Caligiuri M Eds. Kenneth Kaushansky, et al.eds. Williams Hematology, 9e New York, NY: McGraw-Hill .

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